Harry P. Erba, MD, PhD: Hello and thank you for joining this OncLive®Peer Exchange® titled “Management of Myeloproliferative Neoplasms,” focused on polycythemia vera and myelofibrosis. Myeloproliferative neoplasms are often challenging to treat and may require years of therapy and follow-up care. Prognosis is dependent on the specific disorder and response to treatment. Today I am joined by a group of my colleagues who are renowned experts in the field of myeloproliferative neoplasm research. During the next 90 minutes, we will discuss evolving research surrounding the treatment of polycythemia vera and myelofibrosis. We’ll talk about treatment approaches and highlight emerging agents.

I am Dr. Harry Erba, and I am a professor of medicine and director of both the Leukemia Program and the Phase I Program in Hematologic Malignancies at Duke University in Durham, North Carolina.

Participating today on our distinguished panel are Dr. Rami Komrokji, senior member and professor of Oncologic Sciences, section head for Leukemia and Myelodysplastic Syndrome, and vice chair of the Malignant Hematology Department at Moffitt Cancer Center in Tampa, Florida; Dr. Ruben Mesa, director of The Mays Cancer Center, the newly named center of UT Health San Antonio MD Anderson Cancer Center, the Mays Family Foundation Distinguished University Presidential Chair, and a professor of medicine at UT Health San Antonio MD Anderson Cancer Center in San Antonio, Texas; Dr. Jamile Shammo, professor of medicine and director of MDS/MPN and Aplastic Anemia Program in the section of Hematology at Rush University Medical Center in Chicago, Illinois; and Dr. Srdan Verstovsek, United Energy Resources, Inc. professor of medicine and director of the Hanns A. Pielenz Clinical Research Center for Myeloproliferative Neoplasms of the Department of Leukemia at MD Anderson Cancer Center in Houston, Texas.

Thank you so much for joining us. Let’s begin. Let’s get started with an overview of the myeloproliferative neoplasms, and I’m going to start with you, Ruben. Just how common are the myeloproliferative neoplasms?

Ruben A. Mesa, MD, FACP: It’s a good question, Harry. Certainly, patients always ask, “Doctor, is this a rare disease?” I view it as neither rare nor common. Overall, it’s a little over 300,000 to 350,000 patients in the United States. The incidence is fairly low. In each of the diseases of myelofibrosis, that’s maybe around 1 per 100,000 per year. In polycythemia vera and essential thrombocythemia, that’s maybe a little bit higher, closer to 2 per 100,000 per year. The prevalence is actually quite a bit higher due to the longevity that these patients really face, particularly with ET (essential thrombocytopenia) and polycythemia vera. Again, compared to other really rare disorders, in aggregate they’re much more common than CML or similar disorders.

Harry P. Erba, MD, PhD: The myeloproliferative neoplasms used to be called the chronic myeloproliferative disorders, and that term was first coined by Dameshek decades ago. What he noticed was a group of diseases affecting his patients that have certain things in common. I think these features are common to all of the diseases we’re going to talk about today: patients will have high blood counts, they will have splenomegaly and symptoms from it, and they’ll have constitutional symptoms. There is also a particular progression to acute leukemia in some of the patients—sometimes more common in CML, sometimes less common—or what we used to call the spent phase, which we now call the fibrotic phase or post-PV or post-ET myelofibrosis.

What was really precedent about the classification by Dameshek was the other thing that the myeloproliferative neoplasms were found to have in common. He hypothesized back then that someday, we would find out that all of these disorders, neoplasms, are classified together because of aberrant signals that govern normal hematopoiesis. That’s exactly what has been found starting with chronic myeloid leukemia in 1960 with the Philadelphia chromosome and then other genetic abnormalities, such as the JAK2 mutation and many others that we’re going to talk about today.

Transcript Edited for Clarity

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Transcript:

Harry P. Erba, MD, PhD: Hello and thank you for joining this OncLive®Peer Exchange® titled “Management of Myeloproliferative Neoplasms,” focused on polycythemia vera and myelofibrosis. Myeloproliferative neoplasms are often challenging to treat and may require years of therapy and follow-up care. Prognosis is dependent on the specific disorder and response to treatment. Today I am joined by a group of my colleagues who are renowned experts in the field of myeloproliferative neoplasm research. During the next 90 minutes, we will discuss evolving research surrounding the treatment of polycythemia vera and myelofibrosis. We’ll talk about treatment approaches and highlight emerging agents.

I am Dr. Harry Erba, and I am a professor of medicine and director of both the Leukemia Program and the Phase I Program in Hematologic Malignancies at Duke University in Durham, North Carolina.

Participating today on our distinguished panel are Dr. Rami Komrokji, senior member and professor of Oncologic Sciences, section head for Leukemia and Myelodysplastic Syndrome, and vice chair of the Malignant Hematology Department at Moffitt Cancer Center in Tampa, Florida; Dr. Ruben Mesa, director of The Mays Cancer Center, the newly named center of UT Health San Antonio MD Anderson Cancer Center, the Mays Family Foundation Distinguished University Presidential Chair, and a professor of medicine at UT Health San Antonio MD Anderson Cancer Center in San Antonio, Texas; Dr. Jamile Shammo, professor of medicine and director of MDS/MPN and Aplastic Anemia Program in the section of Hematology at Rush University Medical Center in Chicago, Illinois; and Dr. Srdan Verstovsek, United Energy Resources, Inc. professor of medicine and director of the Hanns A. Pielenz Clinical Research Center for Myeloproliferative Neoplasms of the Department of Leukemia at MD Anderson Cancer Center in Houston, Texas.

Thank you so much for joining us. Let’s begin. Let’s get started with an overview of the myeloproliferative neoplasms, and I’m going to start with you, Ruben. Just how common are the myeloproliferative neoplasms?

Ruben A. Mesa, MD, FACP: It’s a good question, Harry. Certainly, patients always ask, “Doctor, is this a rare disease?” I view it as neither rare nor common. Overall, it’s a little over 300,000 to 350,000 patients in the United States. The incidence is fairly low. In each of the diseases of myelofibrosis, that’s maybe around 1 per 100,000 per year. In polycythemia vera and essential thrombocythemia, that’s maybe a little bit higher, closer to 2 per 100,000 per year. The prevalence is actually quite a bit higher due to the longevity that these patients really face, particularly with ET (essential thrombocytopenia) and polycythemia vera. Again, compared to other really rare disorders, in aggregate they’re much more common than CML or similar disorders.

Harry P. Erba, MD, PhD: The myeloproliferative neoplasms used to be called the chronic myeloproliferative disorders, and that term was first coined by Dameshek decades ago. What he noticed was a group of diseases affecting his patients that have certain things in common. I think these features are common to all of the diseases we’re going to talk about today: patients will have high blood counts, they will have splenomegaly and symptoms from it, and they’ll have constitutional symptoms. There is also a particular progression to acute leukemia in some of the patients—sometimes more common in CML, sometimes less common—or what we used to call the spent phase, which we now call the fibrotic phase or post-PV or post-ET myelofibrosis.

What was really precedent about the classification by Dameshek was the other thing that the myeloproliferative neoplasms were found to have in common. He hypothesized back then that someday, we would find out that all of these disorders, neoplasms, are classified together because of aberrant signals that govern normal hematopoiesis. That’s exactly what has been found starting with chronic myeloid leukemia in 1960 with the Philadelphia chromosome and then other genetic abnormalities, such as the JAK2 mutation and many others that we’re going to talk about today.